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Anomalies of the auditory organ in trisomy 18 syndrome: human temporal bone histopathological study

Published online by Cambridge University Press:  08 March 2006

Tohru Tadaki ,
Ryosuke Kamiyama ,
Hiro-Oki Okamura  and
Iwao Ohtani
Tohru Tadaki
Affiliation:
Department of Otolaryngology, Fukushima Medical University, Fukushima, Tokyo, Japan.
Ryosuke Kamiyama
Affiliation:
Department of Audio-Vestibular Neuroscience, Tokyo Medical and Dental University, Tokyo, Japan.
Hiro-Oki Okamura
Affiliation:
Department of Audio-Vestibular Neuroscience, Tokyo Medical and Dental University, Tokyo, Japan.
Iwao Ohtani
Affiliation:
Department of Otolaryngology, Fukushima Medical University, Fukushima, Tokyo, Japan.
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Abstract

The purpose of this study was to define the histopathological changes in the temporal bone of a fetus with trisomy 18 syndrome, a stillborn due to perosplanchnia. Several anomalies were found including malformation of the auditory ossicles, residual mesenchyme in the middle ear, aberrant tensor tympani muscle, absence of stapedial tendon, aberrant lateral ampullary nerve and wide endolymphatic sinus. The incus body was deformed and separated from the long process by connective tissue and monocrural stapes was noted in the right ear. Three-dimensional reconstruction images provided a clear view of the auditory ossicle malformation. The abnormal findings in our case indicate that ear anomalies in this syndrome might be derived from the component around the first and second branchial arches.

Keywords

Trisomy 18Temporal BoneEar OssiclesAbnormalities
Type
Research Article
Information
The Journal of Laryngology & Otology , Volume 117 , Issue 7 , July 2003 , pp. 580 - 583
Copyright
© Royal Society of Medicine Press Limited 2003

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